Sudireddy Kavya, Amin Minorvi, Odeh Rafy, Svrcek Patrick, Khedr Salwa, Hutchinson Lloyd, Patel Shyam A, Cerny Jan, Pearson Laurie
Department of Medicine, UMass Chan Medical School, 55 Lake Avenue North, Worcester, MA 01655, USA.
Department of Neurology, UMass Chan Medical School, 55 Lake Avenue North, Worcester, MA 01655, USA.
Leuk Res Rep. 2025 Aug 8;24:100534. doi: 10.1016/j.lrr.2025.100534. eCollection 2025.
Myeloid sarcomas (MS) are rare extramedullary manifestations of myeloid progenitor cells occurring with or without acute myeloid leukemia. Peritoneal MS is exceptionally uncommon, with no established treatment guidelines, but treatment has historically consisted of cytotoxic chemotherapy. We present the case of a 78-year-old female with 60% CD34+, HLA-DR+, and CD33+ myeloblasts in ascitic fluid, while bone marrow biopsy demonstrated only molecular evidence of the leukemic clone with , and mutations. Findings were consistent with primary peritoneal myeloid sarcoma. Due to chemotherapy ineligibility, the patient was treated with decitabine and venetoclax. After nine cycles, she demonstrated a complete radiographic response. To our knowledge, this is the first case report of a patient with primary peritoneal MS treated with a combination of a hypomethylating agent and venetoclax.
髓系肉瘤（MS）是髓系祖细胞罕见的髓外表现，可伴有或不伴有急性髓系白血病。腹膜MS极为罕见，尚无既定的治疗指南，但以往的治疗方法包括细胞毒性化疗。我们报告一例78岁女性病例，其腹水中有60%的CD34+、HLA-DR+和CD33+成髓细胞，而骨髓活检仅显示白血病克隆的分子证据，存在 及 突变。检查结果符合原发性腹膜髓系肉瘤。由于患者不符合化疗条件，给予地西他滨和维奈托克治疗。九个周期后，她的影像学检查显示完全缓解。据我们所知，这是首例采用去甲基化药物和维奈托克联合治疗原发性腹膜MS患者的病例报告。
